=periodic fever, aphthous stomatitis, pharyngitis, adenitis.

Fever every 4-6 weeks (periodic). Neutrophil count normal, cf cyclical neutropenia.

Besides mouth ulcers, sore throat and cervical lymphadenopathy, headache, musty smell (!), abdo pain.

Affected children continue to grow normally, are well between attacks, and do not suffer long-term sequelae.

Treatment with steroids or with cimetidine has been effective, and some children have had no further attacks following tonsillectomy (which suggests some relationship with strep infection but not clear).

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